Introduction
Congenital heart disease (CHD) occurs in approximately 0.5-0.8% of
live-born children, with a higher percentage in those aborted spontaneously
or stillborn.1 To detect as many children with congenital
heart disease as possible, including those with mild lesions, very
intensive studies are required which may not be available at all hospitals.
2-dimensional echocardiography with colour Doppler has revolutionized
the diagnosis and management of cardiac malformations. It is a non-invasive
investigation that can precisely diagnose most congenital heart diseases
as well as offer treatment options, whether medical or surgical.
We carried out a retrospective 4-year study to ascertain the incidence
of the various congenital heart diseases in our hospitalized patients.
We also studied the symptom profile, the effect on growth and development
and the immunization status of these patients.
Material and Methods
This was a retrospective study, carried out over a 4 year period (from
Jan 1996 to Dec 2000) in a public hospital in Central Mumbai. A review
of the 8893 admissions over this period revealed 165 cases admitted
for congenital heart disease. Patients were included in the study
if they had clinical, laboratory and echocardiographic proof of congenital
heart disease. Basic details (name, age, sex, religion); symptoms
(breathlessness, cyanosis, cyanotic spells, respiratory tract infections,
failure to thrive, refusal to feed and symptoms related to cardiac
failure) and immunization status were noted in pre-structured formats.
Whether normal or delayed were noted. General examination and cardiovascular
examination in detail were entered. Investigations, viz. X-ray chest,
Electrocardiogram and 2-Dimensional Echocardiography findings were
noted as also the final outcome (survived, expired).
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Results
147 children, from birth to 12 years, admitted to the paediatric wards
with proven congenital heart disease formed the study group. There were
96 males and 51 females, with a male to female ratio of 1.88:1. The
age-wise distribution is shown in Table 1. Maximum children (74) presented
in the first year of life, of which 16 were neonates, 108 patients were
Hindus (73.47%), 38 were Muslims (25.85%) and there was only 1 Christian
patient.
There were 88 cases (59.86%) of acyanotic congenital heart disease (ACHD)
and 59 cases of cyanotic congenital heart disease (CCHD). The distribution
of cases is given in Table 2.
12 patients were asymptomatic and were picked up on OPD basis for their
murmurs (Table 3). The commonest symptom was breathlessness (110 patients),
followed by respiratory infection (66 patients) and failure to thrive
(57 patients), the latter two symptoms were maximally seen in patients
with ventricular septal defect (VSD). 44 patients had congestive cardiac
failure (PDA) and atrial septal defect (ASD) each.
Two cases of endocardial cushion defect (ECD) and 1 each of cardiomyopathy
and total anomalous pulmonary venous return (TAPVR) presented with CCF.
Fifty five patients had history of cyanotic spells, which was commonest
in patients with Tetralogy of Fallot (TOF).
Five of the 16 newborns were immunized. Among the 59 cases of CCHD,
32 were completely immunized, 14 were partially immunized and 13 were
unimmunised. Of the 88 cases of ACHD, 49 patients had received complete
immunization, 27 had received partial immunization and 10 were unimmunised.
An evaluation of milestones showed that only 25/59 CCHD patients (42.37%)
and 45/88 ACHD patients (51.13%) had attained normal physical and mental
milestones. A higher percentage of cases of CCHD had delayed milestones
i.e. 57.63% vs 48.86%.
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It is known that 90% cases of Trisomy 18, 50% cases of Trisomy 21 and
40% cases of Turner’s syndrome have CHD.1 In our study,
16 (10%) of cases of congenital heart disease had syndromes and other
associated somatic anomalies, among which Down’s syndrome was
the commonest (43.75%). Khalil et al noted an incidence of 17.9% of
somatic anomalies, with Down syndrome being the commonest and seen in
9.3% cases. When the outcome was noted, 70/88 cases of ACHD were discharged,
15 expired and 3 went against medical advice. Of the 59 cases of CCHD,
39 were discharged, 14 expired and 6 went against medical advice.
Discussion
Congenital heart disease occurs in 8 per 1000 live births and comprises
one of the major diseases in the paediatric age group.2 CHD
has become an important cause of morbidity and mortality in infancy
and accounts for two-thirds of all major birth defects along with neural
tube defects.3 We carried out this study as there are very
few Indian studies stating the epidemiology of CHD in our country.
Of the 8893 admissions, 165 children were admitted with suspected congenital
heart disease. 147 children ages ranging from birth to 12 years were
proven to have congenital heart disease and formed our study group,
giving an incidence of 1.65%.
Our study showed a male preponderance, which is in accordance with studies
by Chadha et al,4 Bidwai et al,5
and Jain et al.6
There are gender differences in the occurrence of specific heart lesions.
TGA and left sided obstructive lesions are slightly more common in boys
(around 65%), whereas VSD, PDA, ASD and PS are more common in girls.
We also found a higher number of males with TGA and left sided obstructive
lesions (50%). Also, we found that VSD was more common among boys, as
was seen in studies by Chadha et al, Bidwai et al
and Rao et al.7 Our study showed a male preponderance
among ASD cases, which was in accordance with studies by Jain et al,
Vashishta et al,8 Rao et al and Chadha
et al. We found an increased number of males with TOF, which
concurs with the findings of Bidwai et al, while Vashistha
et al found an equal sex distribution and Chadha et al found a higher
number of females. The difference in the gender between our study and
known literature as well as other studies could be due to the fact that
we have included only indoor and echocardiography proven cases.
When considering
the age at presentation, we found that maximum number of children were
picked up in infancy, including 16 newborns, as is also seen in other
studies. In the West, however, there are a higher number of patients
picked up in the neonatal period, which could be due to the fact that
foetal echo-cardiography forms a part of their routine antenatal examination.
In a 5 year study (1979-1984) conducted by Udani et al9 the maximum
number of cases were seen between 5-12 years and lowest in . This may
be due to the fact that increased awareness and better facilities are
freely available now and hence, more children are being picked up at
an earlier age than when the study was conducted by Udani et al 20 years
ago.
On looking at the religion, we had a maximum of Hindu patients (73.47%),
followed by Muslims (25.85%) and only one Christian. This could be due
to the fact that the study was conducted at a hospital that is predominantly
a Hindu populated area and has a scattering of Muslim patients.
When the symptoms were taken into consideration, we found breathlessness
to be the commonest symptom, seen in 110 cases (74.83%), followed by
LRTI in 66 patients (44.89%) and FTT in 57 cases (38.77%).
Breathlessness was the commonest symptom in both cyanotic as well as
acyanotic heart disease. LRTI and FTT were maximally seen in cases of
VSD and these patients had large defects with evidence of pulmonary
hypertension. LRTI was also seen in other patients with a large left
to right shunt, as in ASD and PDA. None of the cases of TOF had LRTI
which is a well known fact.
FTT was seen in 7 of 19 cases of ASD and 3 of 4 patients of TAPVR. FTT
is a major symptom of congenital heart disease, the reasons being low
energy intake, low resting energy expenditure, inadequate food intake,
and malabsorption or feeding difficulty.10,11
Of the 44 cases with CCF, 68.2% were of VSD, 11.36% of ASD and PDA each,
4.54% of ECD and 2.27% each of TAPVR and CMP. There were no cases of
TOF as is a well known fact. Also, none of the cases of pentalogy of
Fallots, Hypoplastic heart or single ventricle had CCF.
Fifty five patients gave a history of cyanotic spells and this was commonest
among patients with TOF (29.1%). Keith et al gave an incidence
of 35%.12
Our study had 88 cases of acyanotic congenital heart disease, accounting
for 59.86% of cases and 59 cases of cyanotic congenital heart disease
i.e. 40.16%. The preponderance of acyanotic congenital heart disease
is in concordance with the results of other Indian and Western studies.4-11
VSD was the commonest congenital heart disease seen in our study (36.73%),
which is similar to other Indian studies. TOF was the second commonest
congenital heart disease, seen in 17.68%, and is in agreement with the
results of other Indian studies,4-8 though is higher than
the incidence recorded in Western studies.1,11,12 ASD was
seen in 12.24% cases in our studies, with other Indian studies recording
an incidence from 3.6-23%.4-8
When the immunization history was looked into, we found that 55.1% cases
were completely immunized while 29.25% cases were not fully immunized
for age. 23 cases (15.64%) were unimmunised, however this included 16
newborns, thereby bringing down the figure to 4.76% for unimmunised
cases. The common reasons stated for incomplete immunization were recurrent
LRTI, FTT and ignorance about the importance of immunisation.
Congenital heart disease has been shown to adversely influence the neuro-developmental
outcome of children though the incidence is not known and we could not
find any other study for comparison. The aetio-pathogenesis includes
chronic hypoxia, acute global and focal ischaemia and infection of the
central nervous system leading to lower IQ, poor motor skills, poor
language skills and cognitive impairment.13 These complications
are higher in patients with cyanotic congenital heart disease as was
seen in our study.
Normal physical and mental milestones for age were seen in 46.9% and
48.09% patients respectively, while 33.3% patients had a delay in both
physical and mental milestones. A higher percentage of cases of cyanotic
congenital heart disease had delayed milestones as compared with acyanotic
congenital heart disease (57.63% versus 48.86% respectively). Table
4 compares our echocardiographic findings with those of other studies.
We had no cases of Aortic valve stenosis or Truncus arteriosus, while
most of our data compare with the other studies.
Mortality is known to be higher in CHD, more so in CCHD, the causes
being subacute bacterial endocarditis, refractory failure, arrhythmias,
sepsis and complex congenital cyanotic heart disease. In our study,
the mortality was 19.73%.
Conclusions
In our study, ACHD comprised 88 cases, of which VSD with or without
associated heart defects accounted for 54 cases (61.36%). Among CCHD,
TOF was the commonest, accounting for 26 cases (17.68%).
These children are often deprived of even basic medical care in the
form of immunization. In our study, 23 patients were unimmunised and
41 patients received incomplete immunization.
Growth and development is markedly affected in cases of CHD. 57.63%
had delayed physical milestones, 48.86% had delayed mental milestones,
while 33.3% had a delay in both; related to disease and physiology,
its complications, presence of associated anomalies, coupled with inadequate
medical care and facilities available.
The mortality rate in our study was 19.73%, the causes being refractory
failure, complex congenital heart disease and sepsis.
Hence, we recommend that all murmurs should be screened unless thought
to be physiological. A cardiac evaluation with echocardiography is advised
in all cases of repeated lower respiratory infections and failure to
thrive. All cases of CHD should be under regular monitoring so as to
permit optimal; growth and development. A high index of suspicion, a
detailed history, physical examination, chest X-ray and electrocardiogram
along with the use of 2- D-Echocardiography helps us diagnose most of
the cases of congenital heart disease and early diagnosis, close monitoring
and timely intervention in cases of congenital heart disease will go
a long way in reducing the morbidity and mortality to a large extent.
Acknowledgement
The authors wish to thank the acting Dean, Dr. GV Koppikar, for
her kind permission in allowing us to publish this article.
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008.
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